Investing in Hearing Research, page 4
contact
N E W S
F R O M
T H E
Guys for Eyes, page 8
Beating Childhood Eye Cancer, page 10
M A S S A C H U S E T T S
E Y E
A N D
E A R
I N F I R M A R Y
Farewell to a Grand Physician
RICHARD L. FABIAN, M.D.
When Richard L. Fabian, M.D.,
announced his retirement effective
Oct. 31, 2004, it was a bittersweet
moment for the Boston medical
community — happiness for him, but
profound sadness at the thought of
F A L L / W I N T E R
2 0 0 4
losing such a brilliant colleague. Since
1986, Dr. Fabian has been the Director
of Head and Neck Surgical Oncology Services at Massachusetts Eye
and Ear Infirmary (MEEI) and Massachusetts General Hospital
(MGH). In 1993, he was appointed director of the MEEI’s Norman
Knight Hyperbaric Medicine Center. This self-described “hardworking
doc” is leaving big shoes to fill as surgeon, clinician, author and
Hope for Hayley
teacher to hundreds of residents.
Hayley Benson is a survivor. At 15
Daniel Deschler, M.D., who has worked at MEEI since 2000, is taking
months of age, she has already under-
over Dr. Fabian’s responsibilities in head and neck surgery. He
gone more medical procedures than
described Dr. Fabian as “a phenomenal continues on page 9 >>>
most people ever experience. Hayley
was
diagnosed
with
hereditary
retinoblastoma, the most common
form of eye cancer in children, when
she was just 6 days old. Thanks to the
Helping People Hear
O N E C E L L AT A T I M E
expertise of Dr. Shizuo Mukai, the medical team at the
Massachusetts Eye and Ear Infirmary, and the proton beam, this
Imagine transplanting adult mouse
little girl has a positive future that includes the gift of sight.
stem cells into a chicken embryo to
see how the cells react. Imagine
For Hayley’s parents, Rob and Elizabeth Benson, hearing the news
enabling a deaf mouse — and eventu-
about their newborn daughter was horrifying, but not totally unex-
ally, a deaf person — to hear. Imagine
pected. Rob Benson had also been treated for bilateral retinoblas-
creating drugs to maintain hearing.
toma at the Infirmary with radiation and chemotherapy. He knew that
Welcome to the research laboratory
he was carrying the mutation in the retinoblastoma gene that caus-
of cell biologist Stefan Heller, Ph.D., a principal investigator at the
es retinoblastoma and that each of his children had a 50% chance of
Eaton-Peabody Laboratory (EPL) at Massachusetts Eye and Ear
inheriting it. Treatment had improved significantly since he was a
Infirmary (MEEI).
child, and the Bensons felt they knew what to expect should one of
their children develop retinoblastoma. continues on page 2 >>>
“I am fascinated with the ear because it is a unique niche for a
young researcher,” said Dr. Heller, who is continues on page 6 >>>
Haley continues from page 1 >>>
“Because of family medical history, Dr. Mukai examined our three
children within a week or two of their births,” Mrs. Benson said.
“Through genetic testing that was done when the children were
newborn, we learned that our sons, Tyler and Jake, did not carry the
gene that predisposes them to retinoblastoma. Hayley, on the other
hand, did.”
Hayley had one tumor in the middle of her left eye and three tumors
in her right eye. Dr. Mukai recommended proton beam therapy
instead of chemotherapy. Getting Hayley into the Northeast Proton
Therapy Center on the Massachusetts General Hospital campus was
a bit challenging because the treatment has become so common and
there are a limited number of pediatric cases that can be scheduled
each day. “I freaked out when we were told she’d have to undergo
chemotherapy because there wasn’t space for her. We made lots of
Infirmary Chairman George J. Rabstejnek
phone calls to get her in and we finally did,” said Mrs. Benson.
presented Karen Burns, president of the Boston
“Everyone there was wonderful to us.”
Cured Cancer Club for Laryngectomees, with a
certificate of appreciation for the creation of a
Five days a week for a month, Mr. and Mrs. Benson drove Hayley
new patient education video which is shown to
from their suburban home to Boston. After her temperature, weight
all who have had their “voice boxes” removed.
and lungs were checked, Hayley was anesthetized and irradiated
Proceeds from five Thomas L. Mulligan, Sr.,
with the beam. “She tolerated the procedure very well and had
Memorial Golf Tournaments were used to fund
no side effects,” said Mrs. Benson. “Hayley would drink her whole
this important video.
bottle of formula when she woke up.”
Dr. Mukai examines Hayley every three months under anesthesia
because tumors can reoccur until she’s about 2 years old. When a
new small tumor was found in the peripheral retina of her left eye in
July, Dr. Mukai froze it by using cryotherapy. She now undergoes
more frequent exams and will do so until no more tumors are found.
Explained Mrs. Benson, “Hayley will be examined this way until she’s
about 3 or 4 years old. Eventually, she’ll be seen in the doctor’s office
contact
is produced by the Office of Development and Public Affairs,
(617) 573-3340. www.meei.harvard.edu
© 2004 Massachusetts Eye and Ear Infirmary
once a year without anesthesia.” Hayley’s course of treatment also
includes an occasional MRI , as patients with hereditary retinoblastoma have a slight chance of developing a brain tumor.
Hayley’s parents are thrilled to have Dr. Mukai treating their daughter
and to have the Infirmary’s world-class medical resources so
> F. Curtis Smith, President
readily available. “Hayley is doing exceptionally well at this point and
> Bruce C. Jordan, Vice President for
we are very optimistic,” Mrs. Benson said. “She is a happy, healthy
Development and Public Affairs
> Mary E. Leach, Director of Public Affairs, Editor
> Allison Barnatchez, Melissa Paul, Anne-Marie Seltzer,
Contributing Writers
little girl who always has a smile on her face. She recently began to
walk and has not exhibited any visual difficulties. My husband and I
are so thankful to everyone who took care of Hayley at the Infirmary
as well as MGH.”
> Eric Antoniou, Public Affairs Staff, Photography
> Marc Harpin, rhumba, Design
Mr. Benson adds, “We have the utmost faith in Dr. Mukai. He’s the
best in the country.”
Please write to the Development Office, Massachusetts Eye and Ear Infirmary, 243
Charles Street, Boston, MA 02114 if you wish to have your name removed from the
list of people who receive fundraising requests from the Infirmary in the future. In
For more information about retinoblastoma, see related story on
the event that you write to us with this request, all reasonable efforts will be taken
page 10. — A.-M. Seltzer
to ensure that you will not receive any fundraising communications from us.
2
The Infirmary was pleased to host the
Massachusetts Lions Eye Research Fund’s
annual awards dinner on July 22, 2004. On behalf
of the Department of Ophthalmology, Dr. Joan W.
Miller gratefully accepted a generous grant of
$175,000 to help further research in ophthalmology
at the Infirmary. Pictured (left to right) are
Wayne P. Smith, the 2003-2004 President of the
Lions Eye Research Fund and Dr. Miller,
Massachusetts Eye and Ear Infirmary Chief of
Ophthalmology and Harvard Medical School
Chair of Ophthalmology.
Rob Benson, with his daughter, Hayley.
For the fourth consecutive year, students from PRISM
designed to help students with an interest in science
(Program for Research Investigation in Science and
and math plan for future careers in these areas.
Math), offered through Harvard Medical School, were
Pictured (above), the students pose with Rich Terry,
given an educational and interactive tour of the
M.S., research engineer (far left) and Rick Lewis,
Infirmary’s Jenks Vestibular Laboratory. PRISM is
M.D., balance expert (far right).
3
Investing in Hearing Restoration
For Louise Tillotson struggling to hear has been a part of her life for
regenerating inner ear hair cells, the loss of which has led to hear-
nearly 45 years. “I remember a pain in my ear and then the diffi-
ing loss in more than 28 million Americans. “The more I learned
culty hearing began,” she said. “I never did get a good explanation
about the stem cell research, the more I felt compelled to help
for it. Perhaps it was a virus or maybe just bad luck. Either way,
accelerate it,” she said. “Dr. Heller and his colleagues at the
I’ve been wearing hearing aids ever since.”
Eaton-Peabody Laboratory are onto something big, and I’m grateful
to be able to be a part of it in some small way.”
Mrs. Tillotson, an eternal optimist, never let the hearing aids slow
(For more on Dr. Heller’s work, see related story, page 1.)
her down. For years, she and her late husband, Neil, crisscrossed
the globe managing his rubber business that made latex gloves, Mr.
In just a few months, renovations will begin to build the Neil and
Tillotson’s invention. “We had a great life, and we never had a dull
Louise Tillotson Laboratory for Cell Biology of the Inner Ear, which
moment,” she said. “I don’t remember staying in any one place for
will be home to the Infirmary’s latest scientific recruits, Albert Edge,
very long, before heading off again to the next destination. It was
Ph.D., and Huawei Li, Ph.D. Drs. Edge and Li bring unique expertise
an exciting time.”
to the effort and will focus on understanding early development of
hair cells and creating cell therapies for regenerating hair cells to
Eventually, Mr. and Mrs. Tillotson began to spend more time
restore hearing.
between Boston and New Hampshire. “I was a frequent patient at
the Massachusetts Eye and Ear Infirmary where I had my hearing
“Like the scientists at the Infirmary, my husband was an inventor.
monitored and my hearing aids maintained,” said Mrs. Tillotson. “I
He believed that anything was possible,” said Mrs. Tillotson. “I
became fascinated with the institution, and was excited by the
know that he would share my enthusiasm for the Infirmary and look
cutting edge research to better understand, treat and prevent
ahead with excited anticipation, welcoming the medical innova-
hearing loss.”
tions headed our way.”
Last year, Mrs. Tillotson joined other Infirmary patients and friends
If you would like more information on hearing research at the
in supporting a major new investment in cell biology aimed at
Infirmary, please contact Melissa Paul at 617-573-4168.
Pictured are the late Neil Tillotson and his wife
Louise. The new lab, made possible by their
generosity, will be called the Neil and Louise
4
Tillotson Laboratory for Cell Biology of the inner ear.
Benefit Golf Tournament for Uveitis a Success
When Scott and Alison Green learned that their 3-year-old son,
Connor, had uveitis (an inflammation in the eye lining), they were
devastated, but made a promise that they would not give up hope for
a cure. They sought out C. Steven Foster, M.D., at the Massachusetts
Eye and Ear Infirmary, to treat Connor. Dr. Foster helped ease the
Greens fears by assuring them he would do anything he could to help
preserve Connor’s sight.
But the Greens wanted to do more. Mr. Green decided to help with
uveitis research efforts and organized the “2004 Cure for Uveitis”
golf tournament.
“The golf tournament is a fantastic way of coping,” Mr. Green said. “You
hear about people whose relative is diagnosed with a disease and they
go on a journey to find a cure. In a small way, that’s what we’re doing.”
The golf tournament enabled the Greens to be involved and to
support uveitis research at Massachusetts Eye and Ear Infirmary.
The tournament was held on Aug. 28 in Northborough, MA, at the
Juniper Hills Golf Course. Sixty-three golfers participated in the
event and more than $12,000 was raised to benefit uveitis research
at the Infirmary. The Greens were thrilled by the turnout and the
enormous support received from the event. “We couldn’t have asked
for a better day,” said Mr. Green, “and it is such a wonderful feeling
President F. Curtis Smith and members of the
to see all these people here helping to support our cause.”
senior management team served cake and ice
cream on Oct. 1, 2004 at a celebration marking
For more information about uveitis research at the Infirmary, visit
the 180th anniversary of the Infirmary's founding.
www.uveitis.org , or contact Melissa Paul at 617-573-4168.
The Friends of Massachusetts Eye and Ear
Infirmary presented checks from funds raised at
the Black and White Ball, held in April 2004, to two
Infirmary researchers to help with their research
efforts: Stefan Heller, Ph.D., and Margaret
DeAngelis, Ph.D. Pictured (left to right) at the
check presenting ceremony are: Evalynne Hajjar,
Sandra Heath, Stefan Heller, Ph.D., Caroline Rocco
(at back), Hilda Jabbour, Jane Denis, Margaret
"Meg" DeAngelis Ph.D., Carole Clark, Ruth Nadol
and Mary Lee Culver. The Friends will hold their
next benefit dinner/dance, which will have a
Western theme, on April 30, 2005 at the Harvard
Club on Commonwealth Avenue. Please save the
date! For information on the gala or to join the
Friends, contact Ruth Doyle at 617-573-3164.
5
Helping continues from page 1 >>>
IT BEGAN WITH A FIRECRACKER
Jim Wiggins traces his interest in hearing to “a very
also an assistant professor in the Department of Otology and
Laryngology at Harvard Medical School (HMS). “The ear is very
complicated and very little is known about inner-ear development.
good firecracker” that was set off too close to his ear.
The result was tinnitus — a ringing in the ears — that
The most common problems in the ear are deafness and the failure
of the organ to self-repair.”
varied in intensity from mild to annoying. A second
effect was a considerable empathy for those who
Humans are born with approximately 15,000 hair cells in each ear,
and deafness occurs when many of them die off. Dr. Heller
suffer from more severe tinnitus and from hearing-
described hair cells as “little microphones that are the primary
related problems in general.
sensory inputs for sound. They vibrate forcefully as energy goes
through the ear. The higher the frequency, the faster the hair cells
vibrate. It’s the high-frequency hair cells that die first.”
This empathy and a hope that his own situation might
someday be improved came together when Mr.
Dr. Heller’s research at the EPL comes at a critical time: hearing
Wiggins saw a link on an Internet “blog,” called “the
loss has grown to epidemic proportions. Half of all Americans over
Drudge Report,” about stem cell research being con-
age 70 have significant hearing problems due to heredity, disease,
medications, long-term exposure to excessive noise, or the aging
ducted at Massachusetts Eye and Ear Infirmary by Dr.
process. While previous generations accepted deafness as they
Stefan Heller (see “Helping People Hear”). He called
aged, today’s seniors consider deafness to be a quality of life issue
the Infirmary’s Development Office. After learning that
and expect to hear until they die.
the “hair cell” research effort needed $1 million to be
Experiments are being conducted at MEEI in a large laboratory
fully funded, he began to look more closely. Within a
designed by Dr. Heller and equipped with a cell culture facility,
month, after several phone conversations with Eaton-
fluorescent microscopes, chicken-breeding incubators, and a confocal microscope shared with the Department of Ophthalmology.
Peabody Laboratory Director M. Charles Liberman,
Dr. Heller’s team includes Huawei Li, Ph.D., a postdoctoral associate
Ph.D., Mr. Wiggins pledged the amount needed to
who is joining the faculty this fall, and Albert Edge, Ph.D., a tissue
complete the multi-year $4.2 million campaign to fund
transplantation expert.
a professorship and build a cell biology laboratory to
study “hair cell” regeneration and protection.
Since being recruited by MEEI in 2000, Dr. Heller has been working
with embryonic and adult inner ear stem cells from mice and, more
recently, from humans. He is hopeful that his research will lead
Mr. Wiggins says he was particularly impressed by the
to the creation of drugs that could, within five or 10 years, be
administered during cochlear implant surgery to slow down the
Lab’s interdisciplinary and collaborative focus, the
scale of hearing research at MEEI, and the emphasis
degenerative processes in the inner ear. “We’re still a ways from a
doctor giving a patient a drug for hearing loss,” Dr. Heller said.
on clinically valuable results. The amount was considerably more than he’d planned on giving, but Mr.
One project involved transplanting adult stem cells from the inner
ear of a mouse into chicken embryos. The mouse cells regenerated
Wiggins says he “was very glad to be able to help
into hair cells in the correct position within the chicken ear. “The
move this forward.” In August, he committed an addi-
chicken cells communicated with the mouse cells, signaling that it
tional sum to support a new stem cell research fellow,
who will work with Drs. Albert Edge and Huawei Li.
was time to generate as hair cells,” said Dr. Heller. “Perhaps this
signal can be identified and developed into a drug that can instruct
a mouse cell to regenerate.”
In an experiment requiring painstaking physical skill, Dr. Heller and
Dr. John Brigande, of Oregon Health & Science University in
6
Portland, are experimenting on a mouse with a genetic deficit that
leads to hair cell degeneration. The mouse develops hearing two
weeks after birth, only to lose it within a couple of days. Dr. Heller
ships mouse adult stem cells to Dr. Brigande, who surgically
implants the cells into the inner ears of mouse embryos. The goal
is to make the deaf mouse hear a little bit. It will realistically take
a few years to learn whether deaf mice can hear after such a
surgery because the success rate of the microsurgical implants is
relatively low.
Dr. Heller acknowledges that the strides he is making would not be
possible without funding from men and women who recognize and
support the mission of the EPL. “Without generous gifts from
donors, I would never have been able to explore novel ideas and
novel approaches to understanding the complexities of the human
ear,” he said. “In particular, it is very difficult to get funding for
Collaborators Huawei Li, Ph.D., (left) and Stefan
Heller, Ph.D.
exploratory research, which is so important to discovering new
approaches to finding a cure for deafness. I am very thankful to the
people who so generously helped us get started at MEEI.”
This past summer, Dr. Heller co-organized a 10-week meeting at the
Marine Biological Laboratory in Woods Hole, Massachusetts, with
four researchers and two postdoctoral associates from around the
country who share his interest in inner ear development and inner
ear cell regeneration. The group strategized about what approaches
would be most productive over the next five years to expedite stem
cell regeneration. Dr. Heller hopes that new projects will arise from
this meeting and that the National Institutes of Health or private
foundations can be encouraged to sponsor future meetings,
perhaps an annual summer course on the latest technology for
post-doctoral fellows and students.
“We hope to publish our results from Woods Hole this fall. One goal
is to make the isolation of adult inner ear stem cells more efficient,”
he said. “With mice, it’s not a problem. You just take another mouse.
In human studies, you only get two or three stem cells per person.
We can’t turn them into the hundreds of thousands of cells that we
need. We experimented on mice this summer and whatever we
learned will be applied to humans.”
Pictured: Cross-section of the organ of Corti of an early
postnatal mouse. Hair cells can be distinguished by
expression of specific markers, for example myosin
VIIA (shown in white) and the mouse Atonal 1 gene,
here visualized using transgenic expression of a
nuclear variant of green fluorescent protein. The hair
Dr. Heller envisions doing research to understand the function of hair
cells as well as their regeneration. Very little is known about how
cells’ mechanosensitive organelles, the hair bundles,
are rich in filamentous actin. Here they are seen in red.
hair cells transduce sound and mechanical stimulation into an electric signal. “There aren’t a lot of cells in the ear. If we grow a lot of
hair cells in a culture, we can study their function as well,” he said.
Dr. Heller says, “I couldn’t find a better place to do my work.
Because the Infirmary is a hospital, one has access to doctors who
work with patients, and that’s a big advantage.” — A.-M. Seltzer.
7
Calendar to Benefit Retina Research
Suzi Reynolds wanted to make a difference. Having watched both
of her parents struggle with severe vision problems, she felt
compelled to find a way to fund eye research. “I was driving to
work one day and I asked myself, ‘What can I do to help,’ and the
idea just came to me, why not produce a calendar with the
proceeds to benefit retina research at the Massachusetts Eye and
Ear Infirmary? It seemed achievable and sounded fun,” said Suzi.
Since that May morning, Suzi has worked at a breakneck pace
recruiting models, a make-up artist, and a photographer to
complete 13 photo sessions of handsome men, one for each month
and a group shot for the cover. Her enthusiasm was infectious, and
everywhere she turned, she found people willing to join in and be a
Lucy and Richard L. Fabian, M.D.
part of it. “The most rewarding part of this whole process so far has
been seeing people come together, to want to help,” she said. “You
never realize until you ask, and I haven’t gotten a ‘no’ yet.”
Producing the calendar has given her whole family — and
especially her mother, Lois Finney — a boost. Three years ago, Mrs.
she could sense just the right time when a little humor would help.
Finney’s vision started to decline and she sought medical attention
My mother’s spirits were immediately lifted,” said Suzi.
in her hometown in Colorado. Suzi did some research of her own,
turning up Dr. Joan Miller at the Infirmary. Since Mrs. Finney’s case
Dr. Miller started treating Mrs. Finney with photodynamic therapy,
is quite complicated with several different problems, she flew to
which has since slowed the progression of the disease. She comes
Boston to consult with Dr. Miller, who has since been appointed
to Boston every six weeks for Dr. Miller to closely follow her
Chief of Ophthalmology.
condition. “I know that Dr. Miller is doing everything possible for
me,” said Mrs. Finney. “I am grateful that she is my doctor and that
“The first time we met Dr. Miller, my mother and I knew it just felt
she has dedicated so much of her life to research to help people
right. She was so knowledgeable and yet so kind and caring, and
like me. I hope that the calendar can help accelerate that research
even further.”
The Guys for Eyes Calendar went on sale in October for $15.00. You
can order yours by contacting Suzi Reynolds at 617-899-1998 or by
email at suzi@guys4eyes.org. Look for the Guys for Eyes Web site
going live soon at www.guys4eyes.org for future calendars and
events to help fight retina disease.
The cover of the Guys for Eyes calendar.
8
Lois Finney and her daughter, Suzi Reynolds.
Fabian continues from page 1 >>>
clinician. He connects with people in the treatment of a challenging
Besides his work in head and neck oncology, Dr. Fabian has served
and horrible disease. Along with a great sense of humor, he’s honest
as the director of the Norman Knight Hyperbaric Medicine Center
and offers so much of himself to his patients.”
at MEEI. Norman Knight is a hero at MEEI and the founder and the
president of The Hundred Club of Massachusetts, an organization
Gayl Perry, a head and neck social worker who has known Dr. Fabian
that takes care of the widows and children of firefighters and police
for 16 years, agrees. “For him, healing is not just about removing
officers who die in the line of duty. He met Dr. Fabian when
a cancer or making an incision or doing something structural,” she
approached for a hyperbaric chamber. “The Hundred Club could not
said. “It’s as much about his relationship with his patients as
fund a chamber for legal reasons, but after hearing some stories
anything else. I’ve seen him with patients whom he couldn’t help in
from Dr. Fabian about the need, I was privileged to write checks over
some circumstances. They never felt abandoned. They knew he
the years for three chambers,” noted Mr. Knight. “Dr. Fabian and his
would be with them throughout whatever they had to face.”
staff were the most complete and well-trained staff that anyone had
ever put together, and he learned just about everything he could
Dr. Fabian’s relationship with patients has always been paramount in
about hyperbaric medicine at his own expense.”
his practice. “Availability, affability and ability always apply, and
that’s what I try to teach my residents,” said Dr. Fabian. “Be humble
It was Dr. Fabian’s dream to introduce hyperbaric medicine at the
and treat a patient the way you would want to be treated.”
MEEI because the nearest units were located in Maine and
Connecticut. “My impetus was improving wound healing and dimin-
During a typical work week, Dr. Fabian would see follow-up patients on
ishing complications for patients who had neck cancer. But we’ve
Mondays. “It does my heart good to see my waiting room full,” he said.
gone way beyond my specialty; we treat divers and diabetics,”
explained Dr. Fabian. “We’re still the dominant referral area, but now
One of those Monday patients is Janice Morrow. Diagnosed with
there are more chambers in Massachusetts, as well as most of the
cancer in her jaw and neck in 1994, Mrs. Morrow underwent
other New England states.”
extensive surgery. Last summer, Dr. Fabian operated on her again.
Mr. Knight “feels personally fortunate to have met someone like Dr.
“I put my faith and hope in his hands; I just felt that everything would
Fabian. He is a man whom a person in my position just grows to
be fine, and it was,” explained Mrs. Morrow. “Dr. Fabian is a very
respect and admire because of his modesty, his overwhelming skills,
positive person.”
his desire to train others, and his desire to train the rest of us in being
humble and accepting more responsibility.”
Mrs. Morrow sees Dr. Fabian every three or four months. “I am
heartbroken that he’s retiring. I will miss him dearly, as I am sure all
For Dr. Fabian, the decision to retire came easily. “When you reach
of his patients will. I wish him great happiness in his retirement,”
a certain age, you have to start thinking about your productivity,
she said.
whether you can maintain the pace, maintain delivering the kind of
medicine that you’ve spent your whole life doing. I also have a family
During his career, Dr. Fabian has held appointments at most of
— my wife, Lucy, six children and nine grandchildren. Lucy has
Boston’s leading hospitals. Charles M. (Carl) Norris, Jr., M.D., the
always been there, always understanding. Her support has been
Surgical Director of the Head and Neck Oncology Program at the
essential and so powerful in allowing me to perform professionally.
Dana-Farber Cancer Institute, has known Dr. Fabian since 1978. “He
But the time has come to concentrate more on personal things.”
gave me my first job, and for that I’ll always be grateful,” he said.
Dr. Fabian would like to be remembered as “someone who passed
Dr. Norris offered some historical perspective on Dr. Fabian’s work:
along a way of practicing medicine that is essential for future
“He was instrumental in expanding the treatment options for
doctors. And the person I have to thank is Dr. Joseph B. Nadol, Jr.,
patients with head and neck cancer. During the 1930s, 40s, 50s, and
the Infirmary Chief of Otolaryngology. He hired me.”
60s, treating this type of cancer was a surgeon’s domain. When radiation came along, it was only recognized as a reasonable treatment
Said Dr. Nadol, “Throughout his tenure, Richard Fabian has been
tool in the 1970s. Now surgery, radiation and chemotherapy stand
extraordinarily generous with his talents and time, not only to his
equal in patient treatment. Dr. Fabian, like me, is a cancer physician
patients, but also to his friends, colleagues and resident staff.
whose tool happens to be surgery. For a surgeon to recommend
Throughout his professional career, high ethical standards have
something other than surgery was groundbreaking. He wasn’t the
marked his activity and all of these qualities have made him an
first physician to do it, but he gave notice through example.”
extraordinary role model for young clinicians. We will miss Richard’s
daily presence at the Infirmary, but wish him well in his serial career
and know that we can depend on him for advice and support for our
specialty and our hospital in the future.” — A.-M. Seltzer
9
Beating Childhood Eye Cancer
Welcoming a new baby into the world is a celebratory occasion.
Retinoblastoma refers to a tumor that develops in the retina in early
But parental joy can quickly turn to devastation if the little one
childhood. Retinoblastoma can be hereditary or non-hereditary.
is diagnosed with retinoblastoma, the most common form of eye
In hereditary retinoblastoma (cases are bilateral or have positive
cancer in children. Fortunately, retinoblastoma is considered one of
family history), a child can develop multiple tumors in both eyes.
the most treatable of the pediatric cancers, partly because it is
Youngsters with non-hereditary retinoblastoma generally develop
confined to the eye and partly because it is often detected early.
a tumor in only one eye. Both forms of the disease arise from a
The disease, which affects approximately 250 children in the United
mutation in the retinoblastoma gene.
States annually, has a mortality rate of less than 5%.
The key to successfully treating retinoblastoma is early detection.
The Massachusetts Eye and Ear Infirmary (MEEI) is known around
A tumor can often be seen in a child’s eyes or it may appear in a
the world for its efforts to combat retinoblastoma through innova-
photograph as a white dot in the eye. At the MEEI, youngsters with
tive treatments and for important research in areas such as early
a family history of retinoblastoma are screened every three months
detection. Leading these efforts is Shizuo Mukai, M.D., a Surgeon
until the age of 3 and annually until age 4 or 5.
in Ophthalmology at MEEI and an Assistant Professor of
Ophthalmology at Harvard Medical School. Every week, Dr. Mukai
Unilateral retinoblastoma is often diagnosed when a child is rela-
sees approximately 25 children, many of whom have the disease.
tively older; the average age being 12 to 18 months. “We don’t know
He likes working with children because they are more challenging
when this form of tumor develops,” explained Dr. Mukai. “By the
and the surgeries are more difficult, and because of his research
time the tumor is discovered, the disease may be at an advanced
interest in pediatric eye cancers.
stage, often necessitating the removal of the eye.”
Treatment for retinoblastoma depends on the size, location, and
nature of the tumors and may include radiotherapy, chemotherapy,
laser treatment, cryotherapy, or surgery to remove the eye.
Treating tumors with the proton beam is considered the optimal
treatment if you need to irradiate an eye, said Dr. Mukai, who led
the ophthalmology team at MEEI in the development and clinical
use of this treatment.
“The difference between proton radiotherapy and conventional
radiotherapy is like that between a laser beam and a flashlight. The
profile of the proton beam is very sharp around the borders," he
explained. “You don’t have the diffuse scattering that you see with
a flashlight. In addition, protons can also be stopped very abruptly,
unlike a laser beam that can blow right through healthy tissue. The
goal is minimizing the exposure of any normal tissue to radiation to
reduce its complications, such as a secondary malignancy in
patients with hereditary retinoblastoma.” For children like Hayley
Benson, the proton beam has been life-saving. (See Hayley’s story
on page 1.)
In addition to treating patients, Dr. Mukai is also working on several
exciting research projects. Eager to make strides in early detection,
Dr. Mukai and Dr. Paul Yates, a resident in ophthalmology at the
Dr. Shizou Mukai (left) and Dr. Jennifer Dearden,
anesthesia resident, examine a child under anesthesia.
Infirmary, are designing a system that uses commercially available
digital cameras to take sharp retinal pictures. “Because the
camera would be relatively inexpensive, it would be available to
more doctors,” explained Dr. Mukai. “For example, a digital photo
10
could be sent to a specialist over the Internet for consultation. It
would make evaluation of suspicious cases easier even in remote
areas of the world.”
With Meredithe Appleberry, Ph.D., a principal investigator at the
Howe Laboratory at MEEI, and Dr. Thomas Lee of Cornell University,
Dr. Mukai is also looking at cells that behave like retinae stem cells
or progenitor cells that were isolated from retinoblastoma tumors.
The team is investigating the function of these stem cells in the
tumors. Are these cells the cells of origin for retinoblastoma? Do
these stem cells in the retina behave like stem cells in cancerous
tissue in other parts of the body? Do these cells help the tumor
grow or do they inhibit it? “Our findings may help us determine
potential new treatment approaches,” he said, “and we will
certainly better understand the disease.”
The Infirmary’s Pediatric Unit nurses are pleased
With Bruce Ksander, Ph.D., at the Schepens Eye Research Institute,
Dr. Mukai is using the immune system to treat eye tumors in a
mouse model. Experiments on mice over the past two years have
stopped the cancer in the eye, reduced the spread of cancer to the
liver, and decreased the mortality rate by 50%. “If you take these
same cancer cells and introduce them into the mouse that survived,
to distribute their new Parent Packs to those
families that unexpectedly find themselves at the
Infirmary overnight with their sick child. The
parent packs were developed when a close
friend of the Cartisser family took this on as her
project. Jeannie Este spent many hours at the
Infirmary supporting Matthew Cartisser and his
the cancer doesn’t grow,” Dr. Mukai said. “This work might have an
parents and decided to solicit donations to
application for a cancer vaccine.”
assemble overnight packs for future patient
families. She said the care was excellent, and the
Believing that advances in retinoblastoma can also be made by
staff very supportive, but it felt distressing to be
bringing together specialists from the clinic and the laboratory, Dr.
caught in the hospital without having a few
Mukai and Dr. Eric Grabowski of Massachusetts General Hospital
essentials. Ms. Este found those she contacted
established the New England Retinoblastoma Group. Twenty clinicians
to be very generous when it came to helping
and researchers meet regularly to discuss new treatment methods
children and families. Lands’ End donated 150
and difficult cases.
canvas bags, Relizon donated printing on the
bags, Finagle a Bagel donated 150 free coffee
Said Dr. Mukai, “We now have a protocol that is region-wide, and
we are investigating new ways to treat youngsters using tumor
therapy.” Retinoblastoma affects between 12 and 20 children in the
Boston area per year.
and bagel coupons, Houghton-Mifflin donated
160 “Curious George goes to the Hospital” books,
Infirmary office products companies donated
paper and pens, and the hospital’s storeroom
provided toothbrushes and toothpaste. Pictured
with a Parent Pack are members of the Cartisser
Dr. Mukai conducts his research with several goals in mind. “We
family: Matthew, John and Debbie.
want to investigate new ways to treat this tumor because even
though mortality is low, there is still significant morbidity,” he
explained. “Early detection is also important — the earlier the better
— as is coming up with an approach that minimizes the morbidity
and complications from treatment, such as secondary malignancies. Hereditary retinoblastoma is a systemic disease; these kids
can get other cancers later on, and depending on their age, different
tissues are susceptible. We want to treat the eye problem, make
sure the patients get screened for other cancers as they grow up,
and be able to treat the tumors without causing other cancers.”
– A.-M. Seltzer
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MEEI Nurses Recognized for Excellence
The Infirmary’s Annual Nurses Recognition Dinner on May 12 culminated with presentations of the Norman Knight Nursing Leadership
and Clinical Practice Awards. Made possible by the generosity of
Infirmary friend, Norman Knight, these awards are given to nurses
who show leadership qualities which inspire others and who demonstrate long-term commitment to the provision of quality care for
Infirmary patients. This year’s Norman Knight Leadership Awards
were given to Linda Belkner, R.N., and Eileen Lowell, R.N. Ms.
Belkner has been at the Infirmary for more than 24 years and has
been a nurse manager for 15 years. Ms. Lowell has worked as a staff
(left to right) Rosaida Shkliew, R.N., Soc Pendon,
nurse on the 9th floor, in Pediatrics, and has been a Clinical Leader in
R.N., Carol Covell, R.N., M.S., Norman Knight,
the Ambulatory Unit for two years. The Norman Knight Clinical
Practice Award recipients for this year are Soc Pendon, R.N., Saida
Patricia Donovan, R.N., Eileen Lowell, R.N., and
Linda Belkner, R.N.
Shkliew, R.N., and Patti Donovan, R.N. Ms. Pendon and Ms. Shkliew
are both Clinical Resource Nurses on the 11th floor, where they
given Charlie Wood Awards for Exceptional Patient Service. The
mentor other nurses. Ms. Donovan has been with the Infirmary since
James Schneider Award went to Ann Cola and Rachna Chawla, both
1983 and is a nurse educator in the Operating Room. In addition, Judy
from Health Information Services. The Rita Kelly Scholarship Award
Kelley, Lead Medical Staff Assistant in Otolaryngology, Michelle
went to Amanda Janiak, Unit Coordinator on the 9th floor Same Day
Hasberry, Communications, and Beth Arnold, Social Work, were
Ambulatory Surgery Unit.
Investing in Hearing Research, page 4
Guys for Eyes, page 8
Beating Childhood Eye Cancer, page 10
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